Geographic, Sociodemographic, and Clinical Factors Associated With Parental Self-Efficacy in Pediatric Patients With Hearing Loss.

OBJECTIVE: To identify geographic, sociodemographic, and clinical factors associated with parental self-efficacy in a diverse cohort of deaf or hard-of-hearing (DHH) children. STUDY DESIGN: Cross-sectional study. SETTING: Tertiary children's hospital. METHODS: Four hundred forty parents of DHH children aged 0 to 17 completed the 25-item Scale of Parental Involvement and Self-Efficacy (SPISE) survey from 2014 to 2022. Residential addresses were geocoded and assigned Area Deprivation Index and Social Vulnerability Index rankings, and univariable and multivariable analyses were conducted using sociodemographic and clinical variables, including sex, race/ethnicity, insurance type, survey language, age at the survey, comorbidities, newborn hearing screening results, and hearing loss laterality and severity. RESULTS: Compared to English and Spanish-speaking parents, Chinese-speaking parents were associated with overall lower parental self-efficacy and involvement (regression coefficient = -0.518, [-0.929, -0.106]), Cohen's d = 0.606) and lower scores on items related to their ability to affect multiple aspects of their child's development and expression of thoughts as well as competency in checking and putting on their child's sensory device. Across univariable and multivariable analyses, besides Chinese language, all other sociodemographic, clinical, and geographic variables were not associated with SPISE score. CONCLUSION: To achieve the best patient outcomes, care teams can use the SPISE to evaluate parental self-efficacy and provide targeted support to parents at risk for having lower knowledge and confidence scores about critical skills necessary to facilitate their child's auditory access and language development. Notably, this study found similar reports of parental efficacy across various sociodemographic, clinical, and geographic variables but significantly lower SPISE scores in Chinese-speaking families.

Differences in Hearing Devices and Speech Therapy Utilization Between Children With Permanent Unilateral Versus Bilateral Hearing Loss.

OBJECTIVES: In this study, we aimed to describe differences in diagnosis and both auditory and speech/language intervention utilization between children with permanent unilateral hearing loss as compared with bilateral hearing loss. DESIGN: A retrospective cohort study was performed of children evaluated in a multidisciplinary hearing loss clinic at a tertiary care pediatric hospital. Children aged 0 to 18 years with either permanent unilateral or bilateral hearing loss were included. RESULTS: One hundred fourteen children with unilateral hearing loss and 268 children with bilateral hearing loss were studied for a total of 382 children. There were no demographic differences between children with permanent unilateral versus bilateral hearing loss. Rates of newborn hearing screening and referred screening results were similar between those with unilateral and bilateral hearing loss. Despite similar rates of referred newborn hearing screening, those with bilateral hearing loss were diagnosed at a younger age (mean 3.6 years, SD 3.8 years) as compared with those with unilateral hearing loss (mean 5.0 years, SD 4.2 years). Children with unilateral hearing loss had similar severity of hearing loss in their poorer hearing ear as compared with children with bilateral hearing loss, yet they were significantly less likely to be fitted with hearing devices (53% versus 78%) or receive speech/language therapy (36% versus 54%) as compared with children with bilateral hearing loss. Multivariate analysis found that bilateral hearing loss and earlier age of hearing loss diagnosis were associated with hearing device use. CONCLUSIONS: Early diagnosis and intervention for childhood hearing loss have a significant impact on a child's educational success and social relationships. However, little is known about differences in diagnosis and resource utilization between children with permanent unilateral hearing loss versus bilateral hearing loss. Children with unilateral hearing loss were diagnosed at a later age and were less likely to utilize hearing devices or speech/language therapy compared with those with bilateral hearing loss, despite having similar severity of hearing loss in the poorer hearing ear. There is a strong body of evidence that children with unilateral hearing loss have improved hearing outcomes with hearing devices, which suggests there is room for improvement in identifying unilateral hearing loss and providing adequate services to optimize educational success. However, speech therapy is generally implemented in response to language delays. Therefore, children with unilateral loss may have lower rates of language delays as compared with those with bilateral hearing loss, thereby explaining differences in speech therapy utilization.

Understanding Barriers to Timely Diagnosis and Intervention Among Immigrant Children With Hearing Loss.

OBJECTIVE: Identify the age at diagnosis and intervention of immigrant and/or non-English-speaking children with hearing loss (HL) and risk factors associated with delays. Identify barriers for non-English-speaking caregivers of deaf/hard-of-hearing children. STUDY DESIGN: Sequential mixed methods. SETTING: Tertiary care center in an urban city. METHODS: The analysis includes descriptive statistics, and 1-way and 2-way analysis of variance of the retrospective chart review. The quantitative study demonstrated foreign-born experienced disparities, so we conducted semistructured interviews on a subset of non-English-speaking families in the cohort that was then thematically analyzed using a human-centered design strategy. RESULTS: We divided 532 children into 3 groups: US-born with English as the preferred language (N = 294), US-born and non-English language preferred (N = 173), and foreign-born (N = 67). The laterality of HL and pure-tone averages were similar among the groups (p = .972 and .071, respectively). Age at diagnosis and time to the intervention were significantly different (39.7, 31.5, 75.8 months, p < .001 and 24.6, 29.2, 48.9 months, p = .001, respectively). Ages at diagnosis and intervention were associated with birthplace (p = .005, p = .0005, respectively) but not preferred language (p = .667, p = .343, respectively). Included in the qualitative interviews were Mandarin- (n = 5), Arabic- (n = 4), and Spanish-speaking families (n = 3). Insights revealed participants' quest for anticipatory guidance and social support, the consequences of cultural stigma, and the complexity of caring for a child with HL in an immigrant family. CONCLUSION: Foreign-born children with HL have significant delays in diagnosis and intervention compared to US-born children. For non-English-speaking parents, the diagnosis of HL presents challenges beyond that of the immigrant experience.

Impact of Genetic Testing on Hearing Interventions.

OBJECTIVE: Clinical guidelines recommend genetic testing when evaluating congenital and late-onset sensorineural hearing loss (SNHL). Genetic diagnoses can provide parents additional information regarding anticipated hearing loss progression, comorbid conditions, and family planning. Additionally, obtaining a genetic diagnosis may increase parental acceptance of hearing loss and subsequent pursuit of intervention. This study evaluates the association between genetic diagnoses and hearing loss intervention. METHODS: We included children ages 0-18 years with SNHL who were hearing aid or cochlear implant candidates but non-users and underwent hearing-loss gene panel testing prior to initiating intervention. Univariate analyses were performed to identify predictors of hearing aid fitting or cochlear implantation. Multivariate logistic regression evaluated the impact of demographic and clinical factors on subsequent intervention. RESULTS: Of the 385 children with SNHL who underwent hearing loss gene panel testing, 111 were included. Median age was 7.5 years. 56% were underrepresented minorities, 71% were non-White, and 71% were publicly insured. Those found to have a genetic diagnosis were 4.6 times as likely to subsequently undergo intervention (p = 0.035). Additionally, bilateral hearing loss and earlier age of genetic testing were associated with increased likelihood of intervention. CONCLUSION: Up to half of children with SNHL are suspected to have an underlying genetic etiology. Children diagnosed with a genetic diagnosis are significantly more likely to subsequently utilize hearing aids or cochlear implantation. This provides additional support for clinical guidelines recommending genetic testing not only due to the impact of prognostication but also on treatment decision-making. LEVEL OF EVIDENCE: 4 Laryngoscope, 133:1982-1986, 2023.

Age related changes in gait variability, asymmetry, and bilateral coordination - When does deterioration starts?

BACKGROUND: Gait pattern coordination is affected by several factors (e.g., neurodegeneration), while aging is known to have a significant negative impact. Various gait parameters, such as gait asymmetry (GA) and stride time coefficient of variation (CV), are widely used in both research and clinical settings in order to evaluate human locomotion. Another parameter is the phase coordination index (PCI), which specifically assesses bilateral coordination of gait (BCG), by quantifying the consistency and accuracy of the anti-phased stepping pattern. OBJECTIVE: In this study we hypothesized that there is a steady deterioration in the bilateral coordination of walking through ageing, and in gait rhythmicity, which would be evident by an increase in the values of the coordination parameters which are examined. METHODS: We analyzed gait cycles of 66 healthy participants in ages between 40 and 85 years which were divided into five age groups (40-44; 45-54; 55-64; 75 +). The participants performed corridor walking (i.e., back and forth) wearing a computerized motion sensor-based gait analysis system. PCI, CV and GA parameters were calculated for the straight-line walking segments. RESULTS: PCI values remained relatively stable between the ages of 40-75 (3.16 ± 1.11%), while in the age group of 75 + years old we observed a significant increase (i.e., deterioration in BCG) in PCI values (5.68 ± 2.01%, p < 0.047). Same pattern was seen for the CV parameter. However, GA was not statistically significantly different between all age groups. CONCLUSION: It appears that PCI and CV are more sensitive measures to detect changes in gait through the aging process. The results suggest that potential screening to detect salient gait deterioration should start from the age of 70. On the other hand, GA may be used to identify neurological impairments if found increased at any age.

Early surgical and audiologic outcomes of active, transcutaneous, osseointegrated bone-conduction hearing device (Osia 2® system) placement.

OBJECTIVE: To assess the outcomes of pediatric Osia 2® System placements. METHODS: We performed a retrospective chart review of primary and revision Osia 2® System surgical cases at two tertiary academic children's hospitals. Operative details and post-operative surgical and audiologic outcomes were recorded. RESULTS: 18 cases were performed on 14 children (mean age: 11.5 years, range 7-16) and included 9 primary surgeries and 9 revisions from BAHA Attract®, Connect® and Sophono® implants. Surgical planning for revision surgeries was complex due to prior incisions, implants, and bony contour. Post-operative aided audiograms revealed pure tone average (0.5-4 kHz) of 26.2 ± 2.5 dB HL (mean ± SD), with no high frequency roll-off (8 kHz aided threshold: 23.8 ± 7.5 dB HL). Two minor post-operative complications were identified. CONCLUSION: Bone-conduction hearing devices (BCHDs) are used to improve access to sound for children with conductive hearing loss, single-sided deafness, and aural atresia. Traditional passive, percutaneous abutment-based and transcutaneous magnet-based surgical BCHDs can be limited by skin complications and high-frequency acoustic attenuation. Recent availability of active, transcutaneous osseointegrated BCHD systems presents potential for improvement on both of these traditional limitations. Initial experience with the Osia 2® System demonstrates overall successful, uncomplicated placement with excellent audiologic outcomes. Revision cases require careful surgical planning. Further follow-up and comparative studies with other BCHDs are necessary to fully evaluate the effectiveness of the Osia 2® System.

Disparities in Speech and Language Delay Among Children With Aural Atresia.

OBJECTIVE: To describe risk factors for speech and language delay in a diverse population of children with aural atresia. METHODS: Retrospective chart review was performed from 2012 to 2020 at UCSF Benioff Children's Hospital to identify children with aural atresia evaluated for speech, language, or auditory skills delays. Ninety-five children with aural atresia, conductive hearing loss, and assessment of speech, language, or auditory skills delay were included. Demographic and clinical data were analyzed to identify predictors of speech and language delay. Cohort and case-control analyses were performed to determine risk factors for delayed intervention, and for ultimate delays in speech, language, or auditory skills. RESULTS: Children exhibited a wide diversity of race/ethnicity, primary home language, geography, and insurance status. Eighty-nine percent had unilateral aural atresia. Forty-eight percent had delays in speech, language, or auditory skills. Most children used hearing aids (84%), had educational accommodations (84%), and received speech therapy (63%). In a univariate retrospective cohort analysis, public-insured ( p = 0.004), non-English speaking ( p = 0.002) and non-white/non-Hispanic children ( p = 0.007) were found to be significantly less likely to be fit with hearing aids in infancy. Children with delays were fit with hearing aids at later ages. In a multivariate case-control analysis, primary home language was a significant predictor for presence of delays [OR, 3.9 (95% CI: 1.2-13.2), p = 0.03]. CONCLUSIONS: Disparities due to insurance type, primary language, and race/ethnicity are associated with delays in hearing aid fitting for children with aural atresia. Earlier, hearing amplification is correlated with reduced risk for speech, language, and auditory skills delay. These findings can help identify at-risk children for targeted support.

Access Challenge Index: A Novel Disparity Measure Predictive of Language Outcomes in Children Who Are Deaf/Hard of Hearing.

OBJECTIVE: To evaluate the effect of demographic disparities on language outcomes in a diverse group of children who are deaf or hard of hearing. STUDY DESIGN: Retrospective cohort study. SETTING: UCSF Benioff Children's Hospital (a tertiary care center). METHODS: Forty-four patients aged <18 years were identified with sensorineural hearing loss managed with a behind-the-ear hearing aid or cochlear implant. Demographic and clinical data were extracted from the medical record. The primary outcome measure was the Preschool Language Scales-5 at least 6 months after intervention. Predictors of language outcome were assessed: hearing level at the time of hearing intervention, cochlear implant status, age of identification and intervention, travel time to site of hearing care, home language, race/ethnicity, insurance type, and Access Challenge Index-a novel measure of educational environment and family support based on the Child Cochlear Implant Profile. Multivariate and univariate analysis assessed predictors for association with intervention and receptive, expressive, and total language scores. RESULTS: Overall 82% of patients had cochlear implants. The median age at hearing intervention was 12 months. The sample was 59% female, 52% non-White, and 61% publicly insured, and 20% had a non-English primary home language. Accounting for multiple demographic and clinical predictors, a high Access Challenge Index score was independently associated with longer time to intervention (P = .01) and poorer language outcomes (P < .001). CONCLUSION: Access Challenge Index-a novel comprehensive measure of educational and family environment-is a strong independent predictor of language outcomes in children who are deaf or hard of hearing.

Racial and ethnic disparities in diagnostic efficacy of comprehensive genetic testing for sensorineural hearing loss.

Understanding racial and ethnic disparities in diagnostic rates of genetic testing is critical for health equity. We sought to understand the extent and cause of racial and ethnic disparities in diagnostic efficacy of comprehensive genetic testing (CGT) for sensorineural hearing loss (SNHL). We performed a retrospective cohort study at two tertiary children's hospitals on a diverse cohort of 240 consecutive pediatric patients (76% publicly insured, 82% non-White) with SNHL of unknown etiology who underwent CGT. Definite and possible genetic diagnoses were assigned for each patient, representing the likelihood of a genetic cause of hearing loss. Associations between diagnostic rates were examined. 3.8 ± 2.1 variants were detected per patient; this frequency did not vary between White/Asian and Hispanic/Black cohorts. Overall, 82% of variants were variants of uncertain significance (VUS). Compared with White and Asian subjects, variants identified among Hispanic and Black children were less likely to be classified as pathogenic/likely pathogenic (15% vs. 24%, p < 0.001), and Hispanic and Black children were less likely to have a definite genetic diagnosis (10% vs. 37%, p < 0.001). The adjusted odds ratio for definite genetic diagnosis in Black and Hispanic children compared with White and Asian children was 0.19. Expanding genetic diagnostic criteria to include predicted deleterious VUSs reduced these disparities between White/Asian and Hispanic/Black children, with comparable molecular diagnostic rates (41% vs. 38%, p = 0.72). However, in silico predictions are insufficiently valid for clinical use. Increased inclusion of underrepresented groups in genetic hearing-loss studies to clinically validate these variants is necessary to reduce racial and ethnic disparities in diagnostic efficacy of comprehensive genetic testing.

Racial and ethnic disparities in genetic testing for hearing loss: a systematic review and synthesis.

Racial/ethnic disparities in the diagnostic efficacy of genetic testing for hearing loss have been described. These disparities may relate to differences in variant classification between different racial/ethnic groups, which may, in turn, derive from disparate representation of these groups in the published literature. We sought to quantify racial/ethnic disparities in the published literature on the human genetics of hearing loss. We conducted a search of PubMed for articles describing single-gene, multiple-gene, or whole-exome sequencing for individuals with sensorineural hearing loss. Data on the included subjects, including race/ethnicity and/or region of origin, a number of subjects tested, and method of testing, were extracted. 1355 populations representing 311,092 subjects from 1165 studies were included. Overall, subjects of European and Asian ancestry were equivalently represented, but those of Latino American, African, and indigenous North American ancestry were significantly underrepresented; over 96% of all subjects in the published literature were European or Asian. Within populations, the majority of subjects derived from a small subset of countries. The observed disparity was greater for multiple-gene and whole-exome sequencing than for single-gene sequencing. These findings illustrate the large disparity in the published literature on the genetics of hearing loss, and demonstrate the need for increased representation of Latino American, African, and indigenous North American populations.